Department of Pediatrics, Division of Hematology Oncology
Enzymes and Coenzymes | Hematology | Hemic and Lymphatic Diseases | Inorganic Chemicals | Nutritional and Metabolic Diseases | Therapeutics
Pyruvate kinase (PK) deficiency is the most common red cell glycolytic enzyme defect causing hereditary non-spherocytic hemolytic anemia. Current treatments are mainly supportive and include red cell transfusions and splenectomy.11 Regular red cell transfusions are known to result in iron overload; however, the prevalence and spectrum of transfusion-independent iron overload in the overall PK deficient population has not been well defined. This analysis describes the prevalence and clinical characteristics of iron overload in patients enrolled in the PK Deficiency Natural History Study (NHS) with a focus on those patients who are not regularly transfused.2
Chelation, Iron Overload, Pyruvate Kinase Deficiency, Red Cell Enzyme Abnormalities, Splenectomy
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Copyright © 2018, Ferrata Storti Foundation. Authors' accepted manuscript posted as allowed by the publisher's author rights policy at http://www.haematologica.org/information-for-authors.
DOI of Published Version
Haematologica. 2018 Sep 13. pii: haematol.2018.196295. doi: 10.3324/haematol.2018.196295. [Epub ahead of print] Link to article on publisher's site
van Beers, Eduard J.; Newburger, Peter E.; and Grace, Rachael F., "Prevalence and management of iron overload in pyruvate kinase deficiency: report from the Pyruvate Kinase Deficiency Natural History Study" (2018). Open Access Articles. 3597.